the purpose of this study was to perform a systematic review regarding clinical and histopathological characteristics, immunopathological findings, and treatment for chronic ulcerative stomatitis (CUS).  articles in English, published from January 1962 up to November 2017, assessing clinical and immunological features, treatment, and follow-up of patientes with CUS, were retrieved from three databases (PubMed, Cochrane Library and SCOPUS). A manual literature search was also conducted. A total of 12 studies met inclusion criteria, therefore, were analyzed in this review. CUS shares similiar clinical and microscopic features to those found in oral lichen planus (OLP) and oral lichenoid lesions (OLL). Hence, direct immunofluorescence (DIF) is indispensable to define a final diagnosis. Due to the poor sample availability in the current literature, it is not possible to accurately confirm the prevalence and features of CUS. in order to better evaluate this condition?s findings, further studies with a greater amount of similar immune-mediated diseases should be performed.